内科学-淋巴瘤-邹善华.ppt

LymphomaZhongshanHospital,FudanUniversityZouShanhua,Definition：AgroupofneoplasticdiseasesoflymphoidtissuesClassification：HodgkinsDisease,HD(HodgkinsLymphoma)NonHodgkinsLymphoma,NHL),GeneralConsiderations,ClinicalFeatures：Painless,progressivelymphadenectasisFever,weightloss,nightsweat；cachexiaHepatosplenomegalyEpidemiology：Incidence：incities/inthecountry;male/female;ageHD811；NHL90%,GeneralConsiderations,Virulogy:EBvirusBurkittlymphoma,HDHumanT-cellLymphotropicVirustype1,HTLV-1AdultT-celllymphoma/leukemia,ATLHTLV-2MycosisFungoidesHelicobacterPyloriMALTlymphomaKaposisarcoma-associatedherpesvirusprimarybodycavitylymphoma,EtiologyandPathogenesis,ImmuneStatesofHost：Immunodeficiency/immunosuppressionMechanism：Antigenicstimulationlymphadenosis(modulation)infiniteproliferationlymphoma,EtiologyandPathogenesis,ClassificationofLymphoma,HD：Reed-Sternberg(R-S)Cell,ClassificationofLymphoma,RyeClassification，1966LymphocytepredominanceNodularsclerosisMixedcellularityLymphocytedepletion,ClassificationofLymphoma,WHOClassification,2001NodularlymphocytepredominanceHodgkinslymphomaClassicalHodgkinslymphomaLymphocyte-richclassicalHodgkinslymphomaNodularsclerosisHodgkinslymphomaMixedcellularityHodgkinslymphomaLymphocytedepletionHodgkinslymphoma,ClassificationofLymphoma,NHL：,ClassificationofLymphoma,NHL：IWF，1982REAL，1994WHO，2001,WorkingFormulation,Low-gradelymphomaA.smalllymphocytic,consistentwithchroniclymphocyticleukemiaplasmacytoidB.follicular,predominantlysmallcleavedcelldiffuseareassclerosisC.follicular,mixedsmallcleavedandlargecelldiffuseareassclerosis,WorkingFormulation,Intermediate-gradlymphomaD.follicular,predominantlylargecelldiffuseareassclerosisE.diffuse,smallcleavedcellsclerosisF.diffuse,mixedsmallandlargecellsclerosisepithelioidcellcomponentG.diffuse,largecellcleavedcellnoncleavedcellsclerosis,WorkingFormulation,High-gradelymphomaH.mmunoblastic,largecellplasmacytoidclearcellpolymorphousepithelioidcellcomponentI.lymphoblastic,convolutedornonconvolutedcellJ.smallnoncleavedcell,Burkittsornon-Burkitts,WorkingFormulation,Miscellaneouscompositemycosisfungoideshistiocyticextramedullaryplasmacytomaunclassifiableother,REALClassification,B-cellneoplasmsI.PrecursorB-cellneoplasm:precursorB-lymphoblasticleukemia/lymphomaII.PeripheralB-cellneoplasmsA.B-cellchroniclymphocyticleukemia/prolymphocyticleukemia/smalllymphocyticlymphomaB.Lymphoplasmacytoidlymphoma/immunocytomaC.MantlecelllymphomaD.Folliclecentercelllymphoma,follicular1.Provisionalcytologicgrades:Ismallcell,IImixedsmallandlargecell,IIIlargecell2.Provisionalsubtype:diffuse,predominantlysmallcelltype,REALClassification,E.MarginalzoneB-celllymphoma1.Extranodal(MALT-type+/-monocytoidBcells)2.Provisionalsubtype:nodal(+/-monocytoidBcells)F.Provisionalentity:splenicmarginalzonelymphoma(+/-villouslymphocytes)G.HairycellleukemiaH.Plasmacytoma/plasmacellmyelomaI.DiffuselargeB-celllymphoma1.Subtype:primarymediastinal(thymic)B-celllymphomaJ.BurkittslymphomaK.Provisionalentity:high-gradeB-celllymphoma,Burkitt-like,REALClassification,T-cellandputativeNK-cellneoplasmsI.PrecursorT-cellneoplasm:precursorT-lymphoblasticlymphoma/leukemiaII.PeripheralT-cellandNK-cellneoplasmsA.T-cellchroniclymphocyticleukemia/prolymphocyticleukemiaB.Largegranularlymphocyteleukemia1.T-celltype2.NK-celltypeC.Mycosisfungoides/Sezarysyndrome,REALClassification,D.PeripheralT-celllymphomas,unspecified1.Provisionalcytologiccategories:medium-sizedcell,mixedmediumandlargecell,largecell,lymphoepithelioidcell2.Provisionalsubtype:hepatosplenicgamma/deltaT-celllymphoma3.Provisionalsubtype:subcutaneouspanniculiticT-celllymphomaE.AngioimmunoblasticTcelllymphomaF.Angiocentriclymphoma,REALClassification,G.IntestinalT-celllymphoma(+/-enteropathyassociated)H.AdultT-celllymphoma/leukemiaI.Anaplasticlargecelllymphoma1.CD30+-celltype2.T-celltype3.Null-celltypesJ.Provisionalentity:anaplasticlargecelllymphoma,Hodgkins-like,WHOClassification,2001,B-cellneoplasmsI.PrecursorB-cellneoplasm:a.PrecursorB-lymphoblasticleukemia/lymphomaII.Mature(peripheral)B-cellneoplasmsaB-cellchroniclymphocyticleukemia/smalllymphocyticlymphomabB-cellprolymphocyticleukemiacLymphoplasmacyticlymphomadSpenicmarginalzoneB-celllymphoma(+/-villouslymphocytes)eNodalmarginalzonelymphoma(+/-monocytoidB-cells)fExtranodalmarginalzoneB-celllymphomaofmucosa-associatedlymphoidtissue(MALT)type,WHOClassification,2001,gHairycellleuekmiahPlasmacellmyeloma/plasmacytomaiFollicularlymphoma,folliclecenterjMantlecelllymphomakDiffuselargecellB-celllymphomaMediastinallargeB-celllymphomaIntravascularlargeB-celllymphomaPrimaryeffusionlymphomalBurkittslymphoma/BurkittscellleukemiaB-cellproliferationsofuncertainmalignantpotentialLymphomatoidgranulomatosisPost-transplantlymphoproliferativedisorder,WHOClassification,2001,T-CellandNaturalKillerCellNeoplasmsI.PrecursorTcellneoplasm:a.PrecursorT-lymphoblasticlymphoma/leukemiab.BlasticNKlymphoma,WHOClassification,2001,II.Mature(peripheral)TcellandNK-cellneoplasmsaTcellprolymphocyticleukemiabT-cellgranularlymphocyticleukemiacAggressiveNKCellleukemiadAdultTcelllymphoma/leukemia(HTLV1+)eExtranodalNK/T-celllymphoma,nasaltypefEnteropathy-typeT-celllymphomagHepatosplenicgamma-deltaT-celllymphomahSubcutaneouspanniculitis-likeT-celllymphomaiMycosisfungoides/Sezaryssyndrome,WHOClassification,2001,jPrimaryCutaneousAnaplasticlargecelllymphomaT/nullcellkPeripheralTcelllymphoma,unspecifiedlAngioimmunoblasticTcelllymphomamPrimarySystemicAnaplasticlargecelllymphoma,T/nullcellT-cellproliferationofuncertainmalignantpotentialLymphomatoidpapulosis,ClinicalFeatures,LabandOtherFindings,PeripheralBloodandBoneMarrowHD：Eosinophilia；RScellNHL：Lymphocytosis;ComplicatedleukemiaLDH，2-MG,Coombstest，globulinRadiologicstudiesChestX-rayCT,MRIRadioactivenuclidePET-CTB-ultrasoundBiopsyImmunophenotypeCytogeneticandMolecularStudies,DiagnosisPainless,progressivelymphadenectasisBiopsySkinLesionBiopsyBoneMarrowAspirationandBiopsyBiopsyofOtherSites,DiagnosisandDifferentialDiagnosis,Stagingoflymphoma,StageDefinitionInvolvementofasinglelymphnoderegionorofasingleextranodalorganorsite(E)Involvementoftwoormorenoderegionsonthesamesideofthediaphragm,orlocalizedinvolvementofanextranodalsiteororgan(E)andoneormorelymphnoderegionsonthesamesideofthediaphragmInvolvementoflymphnoderegionsonbothsidesofthediaphragmwhichmayalsobeaccompaniedbylocalizedinvolvementofanextranodalorgansorsite(E)orspleen(s)orboth(sE)DiffuseordisseminatedinvolvementofoneormoredistantextranodalorganswithorwithoutassociatedlymphnodeinvolvementBsymptomsFever38,nightsweats,and/orweightloss10%ofbodyinthe6months,Stagingoflymphoma,DifferentialDiagnosisLymphadenectasis：ChroniclymphadenitisTuberculouslymphadenitisTumournodemetastasesFever：Septicaemia,Tuberculosis,RheumaticdiseaseExtranodalLymphoma:Othermalignanttumer,DiagnosisandDifferentialDiagnosis,RadiationTherapy60Co,acceleratorlinearDosage:3040GY，34W